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Hippocampal sclerosis and epilepsy surgery in neurofibromatosis type 1: case report of a 3-year-old child explored by SEEG and review of the literature.
- Source :
-
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery [Childs Nerv Syst] 2022 Jun; Vol. 38 (6), pp. 1217-1221. Date of Electronic Publication: 2021 Sep 10. - Publication Year :
- 2022
-
Abstract
- Purpose: Epilepsy associated with neurofibromatosis type 1 (NF1) is infrequent and usually controlled with anti-epileptic drugs. However, in some drug-resistant patients a presurgical evaluation should be considered. Hippocampal sclerosis (HS) is one of the rare causes of epilepsy in neurofibromatosis type 1, which can lead to surgery.<br />Methods: We present a three-year-old child with refractory epilepsy associated with several structural brain abnormalities but normal hippocampi on brain MRI and a heterozygous variant in the NF1 gene (c.2542G > A). A complete presurgical evaluation was performed including stereo-electroencephalography (SEEG).<br />Results: Usual seizures were recorded, and the seizure onset zone was delineated in the anterior hippocampus. Pathological examination performed after a tailored mesio-temporal resection confirmed hippocampal sclerosis, and the child achieved seizure freedom with 2 years of follow-up.<br />Conclusion: This rare pediatric case illustrates that NF1 may be associated with early-onset refractory epilepsy secondary to MRI-negative HS, supporting the major role of SEEG in the presurgical evaluation of patients with extended cortical malformations.<br /> (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
- Subjects :
- Child
Child, Preschool
Electroencephalography
Hippocampus diagnostic imaging
Hippocampus pathology
Hippocampus surgery
Humans
Magnetic Resonance Imaging adverse effects
Sclerosis etiology
Sclerosis pathology
Seizures complications
Treatment Outcome
Drug Resistant Epilepsy diagnostic imaging
Drug Resistant Epilepsy etiology
Drug Resistant Epilepsy surgery
Epilepsy etiology
Neurodegenerative Diseases
Neurofibromatosis 1 complications
Neurofibromatosis 1 diagnostic imaging
Neurofibromatosis 1 surgery
Subjects
Details
- Language :
- English
- ISSN :
- 1433-0350
- Volume :
- 38
- Issue :
- 6
- Database :
- MEDLINE
- Journal :
- Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
- Publication Type :
- Academic Journal
- Accession number :
- 34508273
- Full Text :
- https://doi.org/10.1007/s00381-021-05343-0